Question about ET and progression

[Mamab83]

I’m curious how rare progression really is? I seem to see many people who have progressed which surprises me bc all I’ve ever read says it rarely happens. I’m hoping it’s not as common as it seems in message boards and Facebook groups. I’m triple negative and not sure if it’s more, or less, likely to happen to me. I’m also a bit worried bc I’m 36, so does that mean it’s inevitable that eventually it will happen?

[Rae25]

I wonder the same too. I’m 37 and was diagnosed ET Jak2+ at the end of last year.

[Mamab83 in reply to Rae25]

Last year meaning 2019? If so, I was diagnosed December 2019 after finally getting a bone marrow biopsy. My platelets were first discovered high in 2018 after a normal CBC - my first ever, so I have no idea how long they’ve been this high. Luckily I feel good seems a lot of “young” people pop up in these forums as being diagnosed for an issue that was primarily for those over 60 :/

[Rae25 in reply to Mamab83]

Hi Mamba, yes, I was diagnosed in November 2019 after having my bone marrow biopsy in September 2019 - not something I’d like to have again! My platelets were noted as out of range around April 2019. I knew nothing of MPNs until diagnosed so have been learning a lot...

[Paul123456]

Progression curve is exponential, increasing with age. One article said that on average over 60’s have double the progression rate of under 60’s. Hence you are at the best end of the curve.

I posted a Singer prognosis predictor about a month ago if you check my recent posts. I’ve not done so but would be interesting to plug in different ages and see the difference

The most important thing to remember is that understanding of MPN is also increasing exponentially! The difference between now and 10 years ago is enormous. I try to read the latest research (I can just about understand the occasional headline) and I promise you we are living in very exciting times.

My game plan is stay as healthy as possible for next 5 years (exercise/diet) by which time I’m expecting major advances. Not just drugs to delay/regress MF but SCT advances should this become necessary

[Mamab83 in reply to Paul123456]

Your game plan is very similar to mine I like it!

[FG251 in reply to Paul123456]

Does that mean that someone aged, say, 59 who has just been diagnosed has a higher chance of progression than, say, a 35-yr-old who will have had ET for 25 years by the time they reach 60?

[cmc_ufl in reply to FG251]

The amount of time you have had the disease is the strongest risk factor for progression, far greater than simply age at diagnosis. In other words, a 35 year old who had a MPN for 25 years (now 60) has a significantly higher risk of progression at age 60 than someone initially diagnosed at age 60.

This is the reason “most” people with ET have a “normal lifespan”. Because most people are diagnosed 60+, their disease, most of the time, won’t have time to progress before they reach their expected lifespan. However, for people diagnosed young, they face a much different set of circumstances.

Generally speaking, progression of ET/PV is very rare in the first decade (<10%), uncommon in the second decade (10-20%) unlikely in the third decade (20-40%). These are approximate values for MF progression, which are dependent on many factors, including mutation status, overall health, treatment regimen, etc.

[Aneliv9]

I was wondering to. 36 years old. My heam told me a lifetime risk of 15%. But just like you, it seems bigger to me.

[Mamab83 in reply to Aneliv9]

Our hemo would know so I’m gonna just hold faith they are correct! Lol

[fee13]

I think a lot of people are diagnosed with just blood results and there can be quite a few people misdiagnosed without having a bone marrow biopsy to verify so "progression" could actually be what people had to begin with.

[Mamab83 in reply to fee13]

Hmmm I had never thought of that before.. good point. Glad I had a BMB to always compare to

[PhilReade in reply to fee13]

Hi I have E/T jak 2 positive I’m under the Christie hospital had bone marrow test 2013 and one 2014 both negative then after a splenectomy in June 2014 my platelets went through the roof had an angina attack march 2015 and then a haematoma a month later then tested for Jak2 and E/T June 2015 came back positive

I’m 64 I’m on hydroxycarbamide 1500 mg daily and still had haemateura DVTs and a TIA everyone is different but I eat healthy and now my bloods are more balanced my platelets are always 400-500 but the Professor I’m under is happy

Just keep positive eat healthy I take many vitamins to which helps

[Tico]

Hi Mamab83, no it is not inevitable that you will progress and at 36 you have a lot of living to do! You will always see different people on other sites and message boards with their opinions who have not bothered to do their own research.With et,you can live a normal lifespan if you have no complications such as a bleed or clot. Progression is rare and only a small percentage will progress. I suggest you have a look have a look at the Mpn site and do you own research and above all else stop worrying! Atb,tina.🤗

[Ebot]

Hi!

When you say “I seem to see many people who have progressed ..” I just wonder where you are ‘seeing’ them?

I suspect Forums and FB groups like this by their very nature aren’t entirely representative of the MPN population. I was struck at the recent London Patient Day by the proportion of people attending who were diagnosed with, or had concerns about, MF and disease progression. And it certainly was a topic that came up a lot not least because it’s an area in which enormous advances in understanding and intervention are being made. And if you have MF I suspect you might be far more vocal and visible because of the immediacy of the condition.

Furthermore, there are people like me who were originally diagnosed with ET or an unclassified MPN who are later told we have PV. However, in my case - and others - the disease hasn’t ‘progressed’ it has just taken time to emerge definitively.

The reality is, statistically very few people with MPNs progress to MF and even fewer to AML. And understanding about who is at risk and what might be done to manage and / or reduce risk is increasing all the time.

The important thing is, that the risk of things associated with MPNs, like thrombosis, which can really impact on, and shorten, your life are being minimised and managed. As others have said, you have a lot of living to do.

[Mamab83 in reply to Ebot]

that's interesting also.. Hadn't thought about that as well. Yes, I meant through forums and facebook. Especially facebook seems so many have progressed, or are starting to progress. And it may still be a small number, but it appears larger than the odds. What you said makes sense though, I think a lot of people with ET don't comment or think much of it so those aren't heard from as often.

[Hydrox]

Makes me wonder whether I should ask for bmb as I was diagnosed 8 years ago with et Jack 2+ by blood test only. I am 72 now and take hydroxy + aspirin. The last 2 years I seem to have slowed down a lot ie get tired quicker and sleep a lot more. Trouble is I dont know whether it's my age or the illness. The last 2 years I have been monitored by a specialist nurse only, so any concerns have to go through her, and she tells me to see my gp. Would be beneficial to speak to the heamtologist occasionally, I think.

[Mamab83 in reply to Hydrox]

I'm surprised to hear this! From everything I've read the ONLY way to 100% diagnose ET is through a bone marrow biopsy. Blood test alone cannot determine if it's ET or something else. Also, you want a BMB so you can see if fibrosis is happening and then can compare it later if blood work changes.. I would absolutely ask for a bone marrow biopsy ..

[Hydrox in reply to Mamab83]

Thanks for that, I have appointment next week so will ask and see what happens. Will let you know.

[paintbox in reply to Mamab83]

When I was diagnosed with ET JAK2+ in Nov 2018 I was told that because I tested positive for the JAK2+ gene mutation, this signified a clear diagnosis of ET and therefore a BMB was not required. I was also told that the BMB was required to confirm diagnosis in patients, suspected of having ET on the basis of elevated platelet count, but in whom there was no clear gene mutation. I'm seeing my haematologist next week so I'll see what he has to say. I hope you stay well.

[Mamab83 in reply to paintbox]

From everything I’ve read a bmb is required to 100% diagnose.. also I think it’s important to have a starting point for if there is any fibrosis In your marrow - something to compare it to if symptoms change ... good luck to you! Let me know what they say!

[Tico in reply to Hydrox]

Hi Hydrox, I was horrified to read you only ever see a specialist nurse and she tells you to see you Gp with any concerns you have! Considering the majority of Gp's know very little or nothing in some cases would leave me feeling very uneasy about my care at that particular hospital. I would explain to my Cns my concerns and insist on seeing an Haematologist,explaining my Gp's knowledge of rare blood conditions is limited and you are not happy with your treatment at the hospital. If nothing changes,than I would seriously think of asking for a referral to another hospital.Atb,tina.🤗

[Hydrox in reply to Tico]

It see ms to be the norm here, I am in Teesside , UK, that once your illness is considered to be stable, as the heamo put it, then you are seen 3 monthly by a specialist nurse and pick up your meds from her or him. However, I thought that a heamo visit occasionally would have been included. This is not the case as I have not seen him for over 2 years. In a way I am pleased to be deemed as stable, in another I do have concerns that I am not being monitored enough and wonder sometimes if they could be missing something. Especially when you read this forum and hear about other people getting their spleen checked having bmbs and discussing any concerns we may have, makes me think we are being short changed as it were.

[ChelseaF]

I was given the 15% figure and I question whether it changes if you are diagnosed younger. It seems like more decades with the disease mean more opportunity for progression . I was only officially diagnosed last year but my records indicate I have had this since I was 27 at least. I am 41 now. I take it as good news that I have not progressed but my symptoms certainly have so it’s a concern.

[Mamab83 in reply to ChelseaF]

I’m sorry to hear your symptoms have gotten worse. Hopefully they ease up a bit for you soon

[ChelseaF in reply to Mamab83]

Thank you. I am actually feeling pretty well since starting hydroxyurea. Prior to that things were getting progressively worse and my quality of life was awful. I still have symptoms on HU but it’s so much better than off treatment.

[Anag]

Hi Mamab,

I was diagnosed 28 months ago, but have had ET for 10 years. I’m 54. It is absolutely clear that my thrombocytes were climbing steadily that whole time. From 50-70,000 yearly. I have at least 2 blood tests from each year due to my thyroid condition. That my doctor didn’t ring alarm was shocking. I was diagnosed at 823, when I had a TIA! Without meds I’m probably at 1,000 now. As I’ve learned some stay stable for many years, some jump up and down, some increase slowly. Everyone is different. Like you, I try to stay as healthy as possible and am doing many things to free my body of toxins, metals, parasites, etc. My health is very good now. Better than with 40. 🙂 Anag.

[Pte82 in reply to Anag]

Are you being seen by an oncologist or MPN specialist? I hope so. ET is like a coal mine fire. It may smolder for years and when it makes it's presence known it may have progressed to a terminal stage. I encourage you to learn about JAK2 inhibitors like liposomal vitamin C, liposomal curcumin, tocotrienol from both red palm oil and annatto and astaxathin. You'll find selenium and vitamin K2 MK4 & MK7 in addition to boron and magnesium are often over looked. B complex is another to incorporate. Finally quercetin and berberine are going to serve you well. But do get a specialist to pin down your current diagnosis.

[Anag in reply to Pte82]

Of course I have a diagnosis. I have jumped around through 4 heomotologists and left them after they could no longer quench my thirst for getting answers. I don’t have Jak2 I have CALR. My 5th haemo is amazing and accepts all the alternative stuff and the supplements I’m doing. (I have 1300 pages of notes😁) it’s fascinating. But, my blood work still jumps. That’s how I am. Vitamin K made my thrombos absolutely explode as did papayas and sweet potatoes. I do a great Vit C with l-glutathione, manganese,zinc, OPC, and quercitin supplement daily. I take a CoQ10 supplement that has magnesium and Hawthorne in it (incl l-carnitine, Taurine, Quercitine, Alpha lipoic acid, resveritrol, Astaxantin). What a difference in my energy level.

I was taking an amazing B complex in No ember and December, but that made my thrombos explode, as well. I stopped and they are back down. We always have to be careful WHEN we take supplements. Many are strong antioxidants and literally take our meds out of our body before they have a chance to work. ie. my med Anagrelide has a half life of 1.3 hours, therefore I must wait 3 hours before taking antioxidants and 2 hours before taking the next pill. Since the Anagrelid bothers my heart, I must separate the pills throughout the day instead of taking 2 and 2 I Take them separately. This makes taking supplements tricky.

I will definitely look into the other names you sent me! Thank you! I am on mercury and aluminium detox at the moment. I was waiting to finish taking out my last amalgams (just 2 weeks ago!) before starting on high doseTurmeric. I use it for headaches, knee pain and many other ailments. I only make changes one at a time in order to be able to correctly pinpoint cross or adverse reactions.

I will have another blood test in 2 weeks (monthly since my diagnosis), taper off the detox and then start with the Turmeric. I’m really curious! How do you take it? Also, how do the other supplements influence your illness are you ET JAK2?

Looking forward to your correspondence!

Anag